Refractory Central Nervous System Vasculitis and Gastrocnemius Myalgia Syndrome in Crohn's Disease Successfully Treated with Anti-Tumor Necrosis Factor-α Antibody

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doi:10.1016/j.semarthrit.2008.01.008 Copyright © 2008 Elsevier Inc. All rights reserved.

Brief Report

Refractory Central Nervous System Vasculitis and Gastrocnemius Myalgia Syndrome in Crohn's Disease Successfully Treated with Anti-Tumor Necrosis Factor-α Antibody

Sebastian Ullrich MD, , ne Schinke MD†, Markus Both MD‡, Karl Christian Knop MD§, Coula Kirkiles- MD¶, Wolfgang Ludwig Gross MD† and Lamprecht MD† †University of Lübeck, Department of Rheumatology, UKSH Vasculitis Center and Rheumaklinik Bad Bramstedt, Bramstedt, Germany‡Radiology Department, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, GermanyDepartment of Anathomy II, Center for Experimental Medicine, University Hospital of Hamburg, Hamburg, Germany§Department of Neurology, St. Georg Hospital, Hamburg, Germany¶Yale University, Department of Immunobiology, New Haven, CT Available online 4 March 2008.

Background

Secondary vasculitis represents a rare extraintestinal manifestation of Crohn's disease (CD). Appropriate and prompt diagnosis is often delayed by uncertainties about the relationship of the vasculitic manifestations and CD. Objective

To describe our experience with vasculitis in CD and review the literature with respect to different manifestations and pathophysiological aspects of extraintestinal vasculitic manifestations of CD. Methods

We report 2 new cases of CD with secondary small-vessel vasculitis. We also extensively review the literature (1960-2007) using a broad range of key words related to secondary vasculitis in CD. Relevant publications were evaluated for the number of reported patients and manifestations of vasculitis. Results

Vasculitis is a rare extraintestinal manifestation of CD. Different types of vasculitis affect large-, medium-, and small-sized vessels associated with CD. Common immunologic features include intestinal inflammation as well as an infiltration of γδ-T-cells and/or Th1-type cells into vessel walls. The 2 new cases of secondary vasculitis in CD reported here reflect 2 major types of CD-related inflammatory vascular disorders. The first involves the central nervous system, while the second represents circumscribed Musculus gastrocnemius involvement (so-called “gastrocnemius myalgia syndromeâ€). Successful treatment of refractory secondary vasculitis in CD with an anti-tumor necrosis factor-α antibody is shown for the first time. Conclusion

Vasculitis secondary to CD is an uncommon finding. Therefore, it has to be carefully differentiated from other forms of primary or secondary vasculitis with intestinal involvement. Treatment with an anti- tumor necrosis factor-α antibody may prove a treatment option in vasculitis as an extraintestinal manifestation of CD.

Keywords: vasculitis; Crohn's disease; anti-TNF alpha; Takayasu's syndrome; gastrocnemius; myalgia; syndrome; CNS-vasculitis; T-lymphocyte Abbreviations: ANCA, antineutrophil cytoplasmatic antibodies; CD, Crohn's disease; CDAI, Crohn's disease activity index; CNS, central nervous system; CRP, C-reactive protein; CSF, cerebrospinal fluid; MRI, magnetic resonance imaging; MTX, methotrexate; TNF-α, tumor necrosis factor

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